Adulthood interstitial lung disease due to variants in surfactant related genes: A systematic review.

PulmonologySep 24, 2026 (epub)
Clinical ResearchPulmonologyMedical GeneticsPathology (Anatomic & Clinical)

Claudio Tirelli, Stefano Sanduzzi Zamparelli, Camille Louvrier et al.

✦ AI-curated · Sources linked

In 30 seconds

This systematic review examined the clinical features of interstitial lung disease/pulmonary fibrosis (ILD/PF) in adults with surfactant-related gene (SRG) variants. Analyzing 38 studies involving 162 adults, the review found that age at diagnosis ranged from 19 to 79 years, with familial ILD being common in SFTPA1/2 variants. The predominant histological pattern was usual interstitial pneumonia.

Key findings

  • 162 adults with SRG variants were identified across 38 studies.
  • Age at diagnosis ranged from 19 to 79 years.
  • Familial ILD was common in SFTPA1/2 variants, often linked to lung cancer.
  • Lung transplantation showed favorable outcomes in selected patients.

Why it matters

Understanding the clinical presentation and management of SRG-related ILD/PF is crucial for accurate diagnosis and genetic counseling. Improved recognition can lead to better patient outcomes and tailored treatment strategies.

Source

PubMed · PMID 42784242

Published in Pulmonology. This summary was written by xxcode from the publication's abstract and metadata. It is not peer reviewed and is not a substitute for the original article. For clinical decisions, review the original publication.

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